OVERVIEW
Multiple gastrointestinal polyp syndrome refers to a group of syndromes occurring in middle-aged and elderly people with multiple polyps in the gastrointestinal tract (young-type misshapen or inflammatory polyps), ectodermal abnormalities, and gastrointestinal and neurological symptoms such as severe intermittent diarrhea, abdominal pain, and numbness and tingling in the limbs. The age of onset is 30 to 86 years old, with more men than women, and the ratio of male to female incidence is 1.5:1.
Etiology
The cause of the disease is unknown. No genetic factor has been found, and it has been suggested that it may be related to the lack of delayed immune response in the small intestine. Pathologically there is a marked mucosal inflammatory reaction in the gastrointestinal tract, most pronounced in the stomach and small intestine. Polyps are most common in the duodenum and more common in the terminal ileum, and polyps can vary from a few millimeters to 3 centimeters in diameter, with wide variations.
Symptoms
Clinical manifestations are most prominent in diarrhea, which may include abdominal discomfort and anorexia, nausea, vomiting, and abdominal pain. Diarrhea is dilute watery stools, 5 to 7 times a day, and there may be bloody stools or steatorrhea and malabsorption syndrome. Ectodermal abnormalities, which usually appear weeks to months before gastrointestinal symptoms, are manifested by a change in the color of the fingernails (toenails), which may be brown, white, yellow, or black; the surface of the nails is scaly, wrinkled, or spatulate, and there may be atrophy and thinning, splitting, loosening, and peeling. Skin pigmentation, brown spots, ranging from a few millimeters to 10 millimeters in diameter. Neurological symptoms may include numbness and tingling of the limbs, some have loss or loss of taste and smell, and individuals have epileptiform seizures or even fainting. In addition, a significant number of patients develop malabsorption syndrome, protein-losing enteropathy, and therefore have clinical symptoms such as malnutrition, hypoproteinemia, vitamin deficiency, and swelling and anemia. Common complications of the disease include gastrointestinal bleeding, infection, intussusception, carcinoma and thrombosis.
Examination
1. Symptoms
Gastrointestinal symptoms and manifestations of ectodermal abnormalities, mainly diarrhea and abdominal pain.
2. X-ray barium gastrointestinal imaging
To detect multiple polyp images.
3. Gastrointestinal endoscopy
Diffuse multiple polyps are found.
Diagnosis
Diagnosis can be made on the basis of history, clinical symptoms and relevant examinations.
1. Clinical manifestations
Gastrointestinal symptoms and ectodermal abnormalities, mainly diarrhea and abdominal pain.
2. X-ray barium gastrointestinal imaging
Finding multiple polyp images.
3. Gastrointestinal endoscopy
Diffuse multiple polyps are found.
Differential diagnosis
This disease should be differentiated from hereditary gastrointestinal polyposis with mucocutaneous hyperpigmentation, hereditary colon polyp syndrome, Turcot syndrome, Gardner syndrome and other gastrointestinal polyposis, which are not associated with ectodermal abnormalities, which can help to differentiate.
Treatment
The disease is mainly treated with symptomatic and supportive therapies, such as correcting the imbalance of water, electrolyte and acid-base balance, supplementing all kinds of nutrients, preventing and controlling infections, as well as applying antidiarrheal and analgesic drugs. In addition, treatment with corticosteroids can improve the condition and reverse the ectodermal abnormality, or even completely remit it. Surgical treatment is only used for polyps with malignant transformation, complication of gastrointestinal bleeding, intussusception, intestinal obstruction and so on, which are ineffective by internal medicine treatment.